We report a case of a 62-year-old man with limbic encephalitis associated with diffuse large B cell lymphoma. He was hospitalized for the assessment of cognitive disturbance and changes in characteristics. Neurological examination revealed disturbance of recall and recent memory. Magnetic resonance (MR) imaging of the brain revealed bilateral atrophy of the medial temporal lobes. Cerebrospinal fluid (CSF) examination revealed mononuclear pleocytosis and elevated protein levels. Herpes simplex virus DNA was not detected in the CSF by the polymerase chain reaction. Immunological investigation of the patient's serum samples showed no evidence of acute infection with herpes simplex virus or cytomegalovirus. Chest computed tomography (CT) revealed a mass lesion on the right hilum of the lung. Therefore, tumor resection was performed. The pathological diagnosis was diffuse large B cell lymphoma. We diagnosed this patient with paraneoplastic limbic encephalitis. Chemotherapy was performed, but the patient's clinical symptoms failed to improve. We investigated 7 previously reported cases of paraneoplastic limbic encephalitis associated with malignant lymphoma in Japan. We suggest that an early diagnosis of paraneoplastic limbic encephalitis with progressive symptoms such as changes in characteristics is important. Early diagnosis and treatment of malignant tumors is desirable to facilitate clinical recovery and improve prognosis.
[Show abstract][Hide abstract] ABSTRACT: Among paraneoplastic syndromes (PNS) associated with malignant hemopathies, there are few reports of PNS of the central nervous system and most of them are associated with lymphomas. Limbic encephalitis is a rare neurological syndrome classically diagnosed in the context of PNS. We report the case of a 81-year-old man who presented with a relapsed acute myeloid leukemia (AML) with minimal maturation. He was admitted for confusion with unfavorable evolution as he presented a rapidly progressive dementia resulting in death. A brain magnetic resonance imaging, performed 2 months after the onset, was considered normal. An electroencephalogram showed non-specific bilateral slow waves. We received the results of the blood screening of neuronal autoanti-bodies after the patient's death and detected the presence of anti-voltage-gated potassium channel (VGKC) antibodies at 102 pmol/l (normal at <30 pmol/l). Other etiologic studies, including the screening for another cause of rapidly progressive dementia, were negative. To our knowledge, this is the first case of anti-VGKC paraneoplastic limbic encephalitis related to AML.
Case Reports in Oncology 05/2013; 6(2):289-92. DOI:10.1159/000351835
[Show abstract][Hide abstract] ABSTRACT: Identification of etiological connections among virtually distinct diseases in a patient may be sometimes challenging. We report a unique case with two B cell malignancies and an inflammatory leukoencephalopathy. Three days prior to admission, the elderly male patient developed fatigue, headaches, recurrent vomiting, memory disturbances, depression and somnolence. Clinical, laboratory and imaging evaluations as well as post mortem histological studies were performed. Simultaneous presence of primary central nervous system B cell lymphoma, temporal lobe inflammatory leukoencephalopathy and multiple (smoldering) myeloma, was revealed by the detailed work up in the treatment-naïve patient. Based on recent data from genomic studies, we propose that a sequential evolution of molecular pathology lead to the co-occurrence of multiple myeloma and primary central nervous system B cell lymphoma in this patient, and interpret the development of the temporal lobe leukoencephalopathy as a likely paraneoplastic complication of smoldering myeloma.
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